Progressive arthropathy in the course of immune tolerance induction failure in a child with hemophilia A and FVIII inhibitor – a case report

Abstract

We present the case of an 18.5-year-old boy with hemophilia A and FVIII inhibitor detected at the age of 8 months. No persistent inhibitor eradication was achieved, despite three attempts of immune tolerance induction (ITI) with temporary inhibitor resolution and subsequent administration of 11 doses of rituximab. In spite of hemostatic treatment, rehabilitation, and radiosynovectomy, the patient suffered from recurrent bleeding episodes into almost all major joints, which resulted in the development of progressive hemophilic arthropathy. Currently, due to high frequency of bleeding episodes, the patient is being treated prophylactically and receives prophylaxis with activated prothrombin complex concentrate (aPCC).